Pulmonary veno-occlusive disease: a rare cause of pulmonary hypertension in systemic sclerosis. Case presentation and review of the literature .
Rom J Intern Med
; 53(2): 175-83, 2015.
Article
en En
| MEDLINE
| ID: mdl-26402988
ABSTRACT
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary arterial hypertension (PAH). Because of the similar clinical picture of dyspnea on exertion and signs of right heart failure, PVOD is difficult to distinguish from idiopathic PAH. However, the distinction is mandatory because PVOD has a worse prognosis and, more importantly, the administration of PAH specific therapy (vasodilators) can precipitate severe acute pulmonary oedema. We present a challenging case of PAH in a patient with systemic sclerosis in whom a marked decrease in functional capacity after the initiation of bosentan therapy led to the diagnosis of PVOD. Management of PVOD patients is challenging and referral for lung transplantation should be done at the moment of diagnosis.
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Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Esclerodermia Sistémica
/
Enfermedad Veno-Oclusiva Pulmonar
/
Hipertensión Pulmonar
Tipo de estudio:
Prognostic_studies
Límite:
Female
/
Humans
/
Middle aged
Idioma:
En
Revista:
Rom J Intern Med
Asunto de la revista:
MEDICINA INTERNA
Año:
2015
Tipo del documento:
Article